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Hematology Reports
Volume 8
Issue 3
10.4081/hr.2016.6562
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Hematology Reports is published by MDPI from Volume 14 Issue 1 (2022). Previous articles were published by another publisher in Open Access under a CC-BY (or CC-BY-NC-ND) licence, and they are hosted by MDPI on mdpi.com as a courtesy and upon agreement with PAGEPress.
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Case Report

Positive Impact of Eculizumab Therapy on Surgery for Budd- Chiari Syndrome in a Patient with Paroxysmal Nocturnal Hemoglobinuria and a Longterm History of Thrombosis

by
Silvia De-la-Iglesia
*,
Hugo Luzardo
,
Angelina Lemes
,
Melissa Torres
,
Maria Teresa Gómez-Casares
,
Naylen Cruz
and
Teresa Molero
Hematology Department, University Hospital of Gran Canaria Doctor Negrín, Barranco de la Ballena S/N, 35004 Las Palmas de Gran Canaria, Spain
*
Author to whom correspondence should be addressed.
Hematol. Rep. 2016, 8(3), 34-36; https://doi.org/10.4081/hr.2016.6562
Submission received: 27 April 2016 / Revised: 28 June 2016 / Accepted: 30 August 2016 / Published: 30 September 2016

Abstract

Paroxysmal nocturnal hemoglobinuria (PNH) is associated with severe end-organ damage and a high risk of thrombosis. Budd- Chiari syndrome, which develops after thrombotic occlusion of major hepatic blood vessels, is relatively common in PNH and has been associated with increased mortality. We report the case of a 46-year-old male with PNH who presented with Budd-Chiari syndrome associated with portal cavernoma, portal hypertension and hypersplenism. In September 2010, the patient suffered gastrointestinal bleeding, hematuria, and elevated plasma lactate dehydrogenase; he started eculizumab therapy with a good response. In October 2012, he developed upper gastrointestinal variceal bleeding and a splenorenal shunt was placed. At the time of writing, the patient remains stable and eculizumab continues to be effective. There is limited data on the use of eculizumab for prevention of hemolysis and its consequences in PNH patients undergoing surgery. Our findings provide evidence for the efficacy and safety of eculizumab in this setting.
Keywords: paroxysmal nocturnal haemoglobinuria (PNH); Budd-Chiari syndrome; thrombosis; eculizumab; surgery paroxysmal nocturnal haemoglobinuria (PNH); Budd-Chiari syndrome; thrombosis; eculizumab; surgery

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MDPI and ACS Style

De-la-Iglesia, S.; Luzardo, H.; Lemes, A.; Torres, M.; Gómez-Casares, M.T.; Cruz, N.; Molero, T. Positive Impact of Eculizumab Therapy on Surgery for Budd- Chiari Syndrome in a Patient with Paroxysmal Nocturnal Hemoglobinuria and a Longterm History of Thrombosis. Hematol. Rep. 2016, 8, 34-36. https://doi.org/10.4081/hr.2016.6562

AMA Style

De-la-Iglesia S, Luzardo H, Lemes A, Torres M, Gómez-Casares MT, Cruz N, Molero T. Positive Impact of Eculizumab Therapy on Surgery for Budd- Chiari Syndrome in a Patient with Paroxysmal Nocturnal Hemoglobinuria and a Longterm History of Thrombosis. Hematology Reports. 2016; 8(3):34-36. https://doi.org/10.4081/hr.2016.6562

Chicago/Turabian Style

De-la-Iglesia, Silvia, Hugo Luzardo, Angelina Lemes, Melissa Torres, Maria Teresa Gómez-Casares, Naylen Cruz, and Teresa Molero. 2016. "Positive Impact of Eculizumab Therapy on Surgery for Budd- Chiari Syndrome in a Patient with Paroxysmal Nocturnal Hemoglobinuria and a Longterm History of Thrombosis" Hematology Reports 8, no. 3: 34-36. https://doi.org/10.4081/hr.2016.6562

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